[Rickets- and/or scurvy-like bone lesions in beta-thalassemia major].
Recently, a new type of skeletal lesions has been described in Cooley's anemia as a possible complication secondary to therapy. In 12 children affected with thalassemia major, who received an intensive transfusional regimen combined with continuous iron chelation therapy (desferoxamine-B: 50-80 mg/kg/day), some radiological abnormalities of the long bones were observed similar to those described in rickets and scurvy. These rickets and/or scurvy-like lesions had never been reported before the introduction of high-dose desferoxamine therapy. The pathogenesis of these lesions is uncertain, but the toxic effect of desferoxamine probably plays an important role in their development. The association of growth retardation and rickets and/or scurvy-like skeletal lesions in Cooley's anemia patients may be used as a valuable clinical criterion in long-term chelation management.
Orzincolo
C
C
Servizio di Radiologia, Arcispedale S. Anna, Ferrara.
Castaldi
G
G
De Sanctis
V
V
Scutellari
P N
PN
Ciaccio
C
C
Vullo
C
C
D000293:Adolescent; D001847:Bone Diseases / Q000000981:diagnostic imaging* / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D011859:Radiography; D012279:Rickets / Q000000981:diagnostic imaging* / Q000209:etiology; D012614:Scurvy / Q000000981:diagnostic imaging* / Q000209:etiology; D013789:Thalassemia / Q000150:complications*
Lesioni scheletriche simil-rachitiche e/o scorbutiche nella beta-talassemia major.